A multidisciplinary (MDT) approach to

Graves Orbitopathy (GO)

Thyroid Eye disease (TED)the most common inflammatory disease of the

orbit

Vickie LeeConsultant Ophthalmic & Oculoplastic Surgeon

One patient’s journey 40+ year old NIDDM

Asian lady

Graves Hyperthyroidism

2014 Thyroidectomy

Normal TFT levels

non smoker

pain

proptosis

loss of left vision

when trying to look up

unable to move her eye

constant double vision

Euthyroid Post thyroidectomy

Uncontrolled NIDDM HbA1c >100

3 day history of intermittent

sudden loss of vision

pain and double vision

Urgent Referral from another hospital Day 1

MRI orbit to

confirm diagnosis

MDT Thyroid eye clinic CXH

Sight Threatening disease left eye Day 2

3 days of high dose intravenous steroids in

Endocrine Day Unit

Optimisation diabetic control Day 3 -5

Day 6Eye Clinic review

Decision for urgent decompression

urgent orbital decompression

Discharge day 8Day 7

No Radiotherapy due to

diabetes

Start mycophenolate

Mmonths

Weekly Intravenous Steroids Day 14

Monthly review in MDT Thyroid eye clinic

with immunosuppression eye clinic Ongoing Rx

Day 1

pain proptosis

transient visual obscurations

unable to move her eye

constant double vision

Day 8

no pain less proptosis

no transient visual obscurations

improved eye movements

no double vision

6 months later

Recurrence of pain proptosis

transient visual obscurations

pain on moving her eye

constant double vision

Recurrence of orbital inflammation despite orbital decompression IVMP &

ongoing immunosuppression with mycophenolate

Decreasing vision increasing pain

Switched to prednisolone & ciclosporin to try to maintain her vision

Poorly controlled diabetes, diabetic eye disease

not suitable for radiotherapy

Graves Orbitopathy GO

Thyroid Associated orbitopathy TAO

Thyroid eye disease TED

no effective medical cure

poor clinical outcome

No animal model

How is immune tolerance

broken?

50% GD disease have clinically

relevant GO

Can worsen despite good thyroid

control

Can blind or cause severe double

vision

Disfigurement & Impact on QOL

usually much worse than clinical

severity

Why are we talking about

Learning Outcomes

1 Aetiology & Pathogenesis

2 Risk Factors and how to modify them

3 Principles of management of Thyroid Eye Disease (includingTEAMed 5 )

4 A case study to illustrate the need for a multidisciplinaryapproach

Aetiology & Pathogenesis

Graves Disease

60-80% of all cases of

hyperthyroidism

Autoimmune condition

stimulating the TSH receptor

Defined typically by

hyperthyroidism, TSH R

antibody or diffuse uptake on a

Tc NM scan

Pathognomonic features, eye

disease, thyroid bruit

9

Muscle & fat expansion

in a confined space

‘Orbital Cushings’

TrAb

EUGOGO Position Statement Perros et al 2017

Estimated Prevalence of GO

1 in 10 will not have any thyroid hormone problems

of these about half will go on to have thyroid hormone problems

about 1 in 10 will only be affected in one eye socket

family history

thyroid control

genderage

smokingRadioiodine

high TrAb antibody(normal <1.75 IU/L

high risk for progression if >8.8IU/L

12

Who is at risk?

12

13

Smoking

13

• Prummel & Wiersinga 1993

• x7 risk of visual loss

• Higher relapse rate on stopping anti-thyroid drugs

• x4 GO progression post I-131

• poorer response to immunosuppression

GO does not necessarily parallel thyroid activity but

important to render euthyroid

Can worsen post radio iodine treatment

40%

20%50%

40%

75% eye and thyroid onset within the same year

Do not give radio-iodine if

there is significant eye

disease

cover high risk groups with

oral steroids

1414

The disease does ‘burn out’

Lasts on average 1 yr in non smokers 2-3 yrs smokers

Reactivation 5-10%

Risk of blindness uncommon 2-5%

Rundle’s Curve

1515

Prevent visual loss

Modifying severity of

residual disease with timely

intervention

17

Assessing Activity & Severity

Sight

threatening

(2-5%)

Optic neuropathy/ corneal

breakdownImmediate treatment

Moderate to

severe

(29-33%)

Not sight threatening but

sufficient impact on daily life

Lid retraction ≥ 2mm

Moderate to severe soft tissue

involvement

≥ 3mm proptosis (corrected

for race/gender)

diplopia

Immunosuppression (If active)

Surgery (if inactive)

Mild

(65-73%)

Minor impact on daily life

Minor lid retraction

mild soft tissue involvement,

<3mm exophthalmos

no diplopia

Lubricants and other simple

measures

EUropean Graves Orbitopathy GrOup clinical classification

GO is self limiting disease but you need to ask for every patient which severity curve are they on ?

where are they on their disease course?

20

Sight Threatening Disease

2-5%

20 20

Dysthyroid Optic neuropathy

DON

Corneal exposure keratopathy

One study found that in patients with optic neuropathy

28% had other co-morbidities causing poor vision

25% CAS <3

33% no proptosis

DON is a Clinical diagnosis

no single gold standard sign

21

Management

Amsterdam Declaration

for Thyroid Eye Disease 2009

• halving the time

from presentation to diagnosis

• referral to a centre of excellence

for optimal treatment of thyroid

disease

• appropriate use of radioiodine

• avoidance of hypothyroidism

• vigorous anti-smoking measures

vs

titration

block & replace

24

Achieve & maintain Euthyroidism

Thyroidectomy

TeaMed

DiaGO20 questions

13 Q patient

7 Q doctor

Vancouver Orbitopathy

Rule (VOR)

Referral to smoking cessation

services

Discourage passive smoking

Do not give radio-iodine to patients with

significant eye disease requiring

immunosuppression

Good evidence that oral steroid cover and

rapid treatment of post RAI hypothyroidism

mitigates risk in patients with low risk of GO /

mild GO

RAICan worsen GO

Prummel & Wiersinga 1993

PREGO demonstrates that

MDT clinic setup improves

time to diagnosis (6 vs 16

months), time from first

symptoms to first

consultation (9 vs 16 months)

UK Multicentre audit (Orbit

2017) demonstrates similar

findings

MDT speeds up GO Diagnosis

Not enough ophthalmolgists work in thyroid Eye

MDT clinics in the UK

There are not enough clinics to accommodate

this recommendation

Number of ophthalmologists BOPSS surveyed who work in Thyroid MDT clinic

Endocrinologist

Squint

+ lid surgery

RadiotherapistOrbital Radiotherapy

Oculoplastic Surgeon

Functional Decompression

surgery

immunosuppression

specialist

Radiologist

DWI MRI

active inactive

Sight saving

urgent orbital

decompression

(ENT/ Eyes )

Thyroid surgeons

Thyroidectomy

Oculoplastic Surgeon

MDT Thyroid Eye Network @ Central Middlesex Hospital (since 2011)

@ Western Eye Hospital (since 2015)

@ Charing Cross Hospital (starting Jan 2018)

MDT Clinic

Mild disease 60%+

3232 32

Tear Supplements + Selenium

33

Moderate to Sight Threatening

Disease

33 33

Second line immunosuppression

eg mycophenolate

radiotherapy

Intravenous steroids

Orbital decompression

surgery

for blinding disease

There is currently no available treatment to

effectively reverse the protruding eyes in the

acute stage

Soft tissue inflammation (70-

80%)

Double vision (55%)

DON (77%)

little change in proptosis

Immunosuppression

reduces

Urgent sight threatening disease

DON unresponsive to IV steroids

Ulceration or infection of the cornea

Rehabilitation

Severe proptosis (disease should be inactive)

Generally post thyroidectomy

Lateral Medial

Floor

Removal of walls of the orbit

to expand the orbital volume

to ease pressure or allow

tissues to settle back into the

orbit

35

Orbital decompression surgery

treats proptosis and can save vision

35 35

• Blindness

• Haemorrhage

• Double vision

• Periorbital numbness

• Sinusitis

• Asymmetry

• globe malposition

• lid malposition

Lateral Medial

Floor

Post single medial wall decompression

36

Potential complications

36 36

A study among 250 consecutive GO patients seen in

an interdisciplinary thyroid-eye clinic in Germany

reports significant occupational disability

36% were on sick leave

28% were disabled

5% had gone into early retirement

3% had lost their jobsPonto et al 2009

Many GO patients are unhappy and

this may not reflect our clinical

impressions of disease activity /

severity

37

Thyroid eye disease affects

QOL

37 37

38

We collect QOL every visit

38 38

only 1 in 5 units

treating TED in

a UK wide survey

collect QOL data

1Orbital Decompression 3 Eyelid surgery2 Squint Surgery

Rehabilitation Surgery improves QOL

Endocrinologist

Squint & lid

surgery

Radiotherapy

Functional orbital

Decompression surgery

Oculoplastic surgeons

immunosuppression

specialist

Radiologist

DWI MRI

active inactive

Sight saving

urgent orbital

decompression

(ENT/ Eyes)

Thyroidectomy

(thyroid surgeons)

OphthalmologistOculoplastic Surgeon

40

MDT approach improves diagnosis

and optimises management

40 40

Endocrinologist

Squint & lid

surgery

Functional orbital

Decompression surgery

Oculoplastic surgeons

immunosuppression

specialist

Radiologist

DWI MRI

active disease inactive disease

Sight saving

urgent orbital

decompression

(ENT/ Eyes)

Thyroidectomy

(thyroid surgeons)

OphthalmologistOculoplastic Surgeon

41

One patient many specialists

41 41

Prevent visual loss

Modifying severity of

residual disease with

timely intervention

CMH Endocrinology

Claire Feeney

Wing May Kong

Pari Avari

Dan Darko

K Muralidhara

SMH Endocrinology

Stephen Robinson

Vassiliki Bravis

CXH Endocrinology

Karim Meeran

Emma Hatfield

CXH Radiotherapy

Sarah Partridge

Simon Stewart

NWP Head & Neck

Mike Perry

Joe Marais

WEH

Rajni Jain

Ahmad Aziz

Rashmi Akishar

Tessa Fayers

special thanks to

MDT Clinic Co-ordinators

Jenny Coelho (CMH)

Toussaint Smith, Ashley Gayle

(Imperial)

CXH ENT

Catherine Rennie

Hesham Saleh

Thyroid Surgeons

Neil Tolley

Fausto Palazzo

CMH Botulinum toxin

clinic Dhannie

Ramacharan